Motor Neurone Disease

Question 1

MND

Classic presentation

Answer 1

• insidious onset
• UMN involvement (increased tone + hyperreflexia)
• LMN: involvement (fasculations)

a painless progressive motor weakness that may begin in the limbs, axial or bulbar muscles and then spreads throughout the body, resulting in progressive disability and eventual death

Question 2

MND

What are some facial features particularly for bulbar MND?

Answer 2

• dysarthria (difficulty speaking)
• sialorrhoea (excessive salivation)
• dysphagia (difficulty eating)

Question 3

MND

How do we diagnose it?

Answer 3

• clinically

Question 4

MND

Different types

4

Answer 4

• amyotrophic lateral sclerosis (most common)
• progressive bulbar palsy
• progressive muscular atrophy
• primary lateral sclerosis

Question 5

MND

What kind of resp failure can result? Why?

Answer 5

Type 2
chest wall weakness

Question 6

MND

What investigations might be done?

Answer 6

Electromyography
Nerve conduction studies
MRI scan of the brain

Question 7

MND

Why do we do a MRI scan?

Answer 7

to rule out other pathologies, such as strokes, MS, parkinsons

Question 8

MND

What is the only licensed medication available that may alter the prognosis?

Answer 8

Riluzole (a glutamate release antagonist)

Question 9

MND

What mendication can help with spastisity?

Answer 9

baclofen, dantrolene or gabapentin